BTX Improves Appearance of Scleroderma ‘En Coup De Sabre’ in 2 Patients, Study Shows

Ashraf Malhas, PhD avatar

by Ashraf Malhas, PhD |

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EHP-101 Phase 2a trial

A study involving two patients has demonstrated the effects of botulinum toxin in improving the appearance of a facial abnormality known as scleroderma en coup de sabre, which is sometimes seen in linear scleroderma.

Localized scleroderma is a form of scleroderma that affects only the skin. Linear scleroderma is a rare form of localized scleroderma characterized by skin and subcutaneous tissue abnormalities found on one side of the body.

Sometimes patients have a facial abnormality called en coup de sabre, or ECDS, a thickening of skin across the face that may look like a sword cut and usually extends from the forehead into the frontal scalp.

Previous studies have shown that the use of the chemotherapeutic agent methotrexate and intravenous corticosteroids can be effective in treating ECDS. But these methods aren’t always successful.

More recently, a study has demonstrated the effectiveness of the toxin produced by the bacterium Clostridium botulinum, botulinum toxin (BTX), in treating the appearance of ECDS.

The study, “Enhancement of the aesthetic outcome of scleroderma en coup de sabre with botulinum toxin injection,” was published in the Journal of the American Academy of Dermatology Case Reports.

Two cases of patients in Saudi Arabia who were treated with BTX were described in the study. The first case was a 33-year-old man who had ECDS for seven years and had been treated with other therapies. He was given two injections of BTX on each side of the ECDS, and the effect was evaluated by taking pictures before the injections and 10 days after.

The patient did not report any side effects, and the treatment resulted in “improvement in the hyperfunctional facial lines and a remarkable decrease in sclerotic changes,” according to the researchers.

The second case was a 29-year-old woman who had ECDS for three years. She underwent treatment with BTX that involved a single injection on each side of the lesion because her muscles were deemed weaker than those of the first patient. When she was assessed at 10 days post-injection, the results were reported to be a “remarkable aesthetic improvement,” the researchers wrote.

BTX has recently received attention for the treatment of cosmetic and non-cosmetic dermatology. It is thought that by blocking the release of the neurotransmitter acetylcholine, BTX causes a relocation of the tense muscles and the observed effect on ECDS.

Although both patients reported being satisfied with the treatment and were willing to repeat it in the future, BTX is not treating ECDS itself, but is having an effect on “the appearance of the patients by reducing the groove induced by ECDS,” the researchers emphasized.